Recently, type 2 inflammation has been recognized as one of the most critical factors participating in the pathogenesis of cystic fibrosis (CF). On the one hand, type 2 inflammation restores tissue homeostasis and contributes to the resolution of inflammation following an injury. On the other hand, type 2 response‐activated immune cells may become dysregulated or chronically activated, causing tissue fibrosis. Among the type 2 cytokine‐driven inflammatory pathways, the transforming growth factor β (TGFβ), interleukin (IL)‐17, IL‐33, and IL‐13 have been identified as essential mediators in patients suffering from CF. Given their critical role, we firmly believe that an adequate comprehension of the type 2‐mediated pathways can identify attractive targets to decrease pharmacologically the inflammation and fibrosis occurring in the pulmonary tissue of patients suffering from CF.